“…Consequently, PPIX-mediated bile duct blockage results in cholestatic liver injury, which can further lead to fibrosis, cirrhosis and liver failure [7–10, 14]. Various treatments have been used to manage EPP-associated liver injury in clinical practice, such as suppressing PPIX production by iron therapy [15] or intravenous administration of hemin [16], reducing PPIX pool by plasmapheresis or hemodialysis [17], interrupting the enterohepatic circulation of PPIX by charcoal [18], increasing bile flow by chenodeoxycholic acid or ursodeoxycholic acid [19, 20], and inhibiting oxidative stress by vitamin E [21]. However, the efficiency of these approaches has not been proved in large numbers of patients and none of them is consistently effective in all cases [5, 9, 10].…”